Increased intracranial pressure is one of the most severe complications with significant mortality in children,so early diagnosis and treatment of this disorder is critical to save the patient's life.This article reviews etiologies,pathophysiology,and general principles of diagnosis and management of increased intracranial pressure.Based on primary diseases and clinical presentations,the goal of therapeutic strategy is to decrease intracranial pressure,avoid neurologic sequelae,and improve the outcome in patients.

Objective To evaluate the efficacy and safety of percutaneous transluminal angioplasty and stenting of intravascular stenting (PTAS) and internal medicine in the treatment of secondary severe stroke in young patients with severe symptomatic middle cerebral artery stenosis.Methods The clinical data of 77 cases with severe symptomatic middle cerebral artery stenosis(ste nosis rate≥70％)confirmed by digital subtraction angiography(DSA) were collected retrospectively in our hospital from January 2011 to June 2015.The patients were divided into PTAS group and medical treatment group,and the data were collected including the modified Rankin Score (mRS score) at admission,the US National Institutes of Health Stroke volume neurological impairments score (NIHSS score)at admission,as well as mRS score,the recurrence of ischemic stroke,death and intracerebral hemorrhage within 1 year.Results The primary end-point rates within 30 days after enrollment in PTAS group and medical treatment group were 0％ and 5.77％ respectively,and the stroke recurrence rates within 1 year were 4.35％ and 13.46％ respectively,The differ ence was not statistically significant(P＞0.05).Meanwhile,there was no death and intracerebral hemorrhage in both two groups.The rates of mRS≤1 were 91.30 ％ and 69.23 ％ respectively in PTAS group and medical treatment group,and the difference was statistically significant (P＜0.05).The rates of mRS≤2 were 95.65 ％ and 84.62 ％ respectively in PTAS group and medical treat ment group,and the difference was not statistically significant(P＞0.05).Conclusion PTAS is safe for the severe symptomatic middle cerebral artery stenosis,and is more efficient in dectasing the risk of recurrent stroke in young population compared with medical treatment.

Objective The objective of this article was to compare patients' dose with electrocardiographic gated mA modulation (ECG) and ECG＆CAREDose 4D mode during coronary MSCT angiography.Methods The research was based on phantom experiment and computer simulation to get the mean value of peak skin dose data and effective dose data respectively and to analyze deterministic and stochastic radiation risk.Results The peak skin dose using ECG mode alone and using ECG＆CAREDose 4D mode with the same image noise level was (87.4±0.9) and (45.9 ± 1.2) mGy respectively.Effective dose was 17 and 10 mSy for ECG mode and ECG＆CAREDose 4D mode respectively.Comparing with ECG mode alone, ECG＆CAREDose 4D mode reduced organ dose of gonad, red marrow, lung, stomach, breast and thyroid by 40.0%, 36.7%, 39.3%, 37.7%, 38.8% and 38.9%, respectively. Conclusion Results showed that ECG ＆ CAREDose 4D mode can reduce radiation dose effectively comparing using ECG mode alone, and that ECG ＆ CAREDose 4D mode should be widely applied ehnically with appropriate initial settings.

Objective To observe the effect of sinomenine(SN) in vitro on nuclear factor-?B(NF-?B) DNA binding activity and nuclear translocation of synoviocytes in collagen-induced arthritis(CIA) rats and explore its antiinflammatory mechanisms.Methods The experimental model of CIA rats was used and synoviocytes were collected.Cells were divided into five groups:normal control,CIA,CIA+10 ?mol/L methotrexate(MTX),CIA+50 ?mol/L SN,CIA+500 ?mol/L SN.Nuclear translocation of NF-?B p65 subunit and NF-?B DNA binding activity of synoviocytes were investigated by fluorescence labelling laser confocal scanning microscopy and electrophoretic mobility shift assay(EMSA) respectively.Results Compared to normal control,significant nuclear translocation of NF-?B p65 subunit was observed and NF-?B DNA binding activity was increased in synoviocytes of CIA rats(P

Objective To investigate elderly patients with thoracic radiotherapy nausea result after risk factors associated with radiation pneumonitis.Methods The clinical data of a total of 440 cases of cancer patients with chest radiation therapy during January 2010-January 2014 were collected retrospectively.Of them,76 cases of radiation pneumonitis after radiotherapy were compared with other patients.The unconditional Logistic regression analysis was used to analyze the relationship of radiation pneumonitis and different factors including smoking,pulmonary dysfunction,combination with chemotherapy,radiation dose,and radiation sites.Results Elderly incidence of radiation pneumonitis was 17.27％.Multivariate Logistic regression analysis revealed the chi-square value of smoking,pulmonary dysfunction,combined with chemotherapy,radiation dose,and radiation sites was significant correlation (x2 =16.936,19.633,11.531,17.133,10.178,P ＜0.05),and the correlation degree was gradually decreased from pulmonary dysfunction,radiation dose,smoking,combined chemotherapy,to radiation site.Conclusions Elderly patients with thoracic malignancies after radiotherapy had more radiation pneumonitis,which was related to smoking,previous chemotherapy PO2 ＜ 80％,combined with chemotherapy,radiation dose ≥ 55 Gy,and low-lung radiation.The correlation degree was gradually decreased from chemotherapy before PO2 ＜ 80％,the radiation dose ≥55 Gy,smoking,combined chemotherapy,to low-lung radiation.

Objective To investigate the molecular mechanism for change in permeability in brain microvascular endothelial cells (bEnd.3) induced by lipopolysaccharide (LPS). Methods Monolayers of bEnd.3 were exposed to LPS,in the presence or absence of exoenzyme C3 transferase. We monitored the monolayer barrier integrity by transendothelial electrical resistance assay (TEER),activity of RhoA by pull down assay,NF-κB by luciferase reporter assay,and F-actin dynamic structure by Rhodamine-phalloidin staining. Results Incubation of monolayers with LPS caused substantial barrier hyperpermeability. Under the had been treated for 3 and 12 h with LPS (P＜0.05). Such effects could be inhibited partly by pretreatment of RhoA inhibitor exoenzyme C3 transferase. LPS activated RhoA and NF-κB at 0.5 h. The C3 transferase could significantly reverse the NF-κB activation (P＜0.05). The F-actin rearrangments displayed in a time-dependent manner and occurred originally after the stimulation of LPS for 3 h,which could be diluted by the pretreatment of C3 transferase as well. Conclusion LPS induces the disruption of F-actin cytoskeleton and brain microvascular endothelial barrier integrity,in part,through RhoA and NF-κB activation. The mechanism underlying this pathophysiological effect of RhoA is to influence the disruption of the F-actin cytoskeleton by regulating NF-κB activites.

Infantile neuroaxonal dystrophy (INAD) is a rare autosome-recessive disease characterized by progressive motor and cognitive regression.The PLA2G6 gene is its causative gene,which encodes calcium-independent phospholipase A2 enzyme (iPLA2-VIA).The diagnosis of INAD is difficult because of its clinical heterogeneity,and the rate of misdiagnosis is high.The purpose of this study is to describe the clinical characteristics,molecular genetics,treatment and prognosis of INAD to improve the acknowledgement of INAD in medical workers and to help make an early diagnosis of INAD.

Four boys (2 months to 8 years old) were diagnosed with autosomal recessive form of osteopetrosis. Symptoms manifested in the first few months of life in 3 patients, and there was family history in 1. Primary symptoms included anemia, thrombocytopenia, hepatosplenomegaly, failure to thrive, recurrent infectious history and macrocephaly. The typical radiological images on plain radiogram were diffuse sclerosis, bone modelling defects at the metaphyses of long bones, "bone-in-bone" appearance, and "sandwich" vertebrae. Bone marrow biopsy showed markedly reduced platelets. Osteopetrosis refers to a group of rare, heritable disorders of the skeleton characterized by increased bone density on radiographs. Diffuse sclerosis leads to crowding of the bone marrow, resulting in anemia and extramedullary hemopoiesis. Hematopoietic stem cell transplantation is employed for the most severe forms associated with bone marrow failure and offers the best chance of longer-term survival.
Child ; Diagnosis, Differential ; Female ; Humans ; Infant ; Male ; Osteopetrosis ; diagnosis ; diagnostic imaging ; Radiography

This patient presented with fever,seizure and bulging fontanelle when he was 6-month-old.According to the investigations,white blood cell (WBC),erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) increased significantly,and Streptococcus Pneumonia grew in both blood and cerebrospinal fluid (CSF).He responded to standard antibiotic treatment poorly even it lasted long enough.At the same time,the inflammation seemed to be over-activated,the WBC level was still elevated,high fever continued.Thus they thought of primary immunodeficiency and sent blood sample for gene panel testing (Sanger sequencing) but got negative result.At last,they added steroid together with anti-tuberculosis drug therapy,his temperature as well as the intracranial pressure became better ever since.At the age of 1 year and 1 month,he got another Streptococcus Pneumonia meningitis,while he was still on anti-tuberculosis drug therapy and tapering off steroid.At this time,he presented with coarse hair,hypohidrosis and delayed eruption of teeth,which strongly indicated Anhidrotic Ectodermal Dysplasia with Immunodeficiency (EDA-ID).NEMO is the most common gene responsible for EDA-ID and locates on X chromosome.It has a pseudogene named IKBKGP which locates downstream of NEMO.IKBKGP and NEMO share 3-10 exons with the homology of 99.8％,which makes it difficult to find out most real mutations within NEMO with Sanger sequencing.Then they performed PCR with the primer starting upstream of the shared exons.Finally,they found out the pathogenic mutation [c.505G ＞ C(p.A169P)] of NEMO,which has been reported.This finding led us to make the right diagnosis as well as the proper treatment and the prognosis for this patient.

Objective: To investigate the clinical features, diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES). Methods: The clinical data of 12 children with FIRES admitted to Xiangya Hospital of Central South University from 2015 to 2018 were retrospectively analyzed. The basic information, clinical manifestations, electroencephalogram, imaging examination, treatment and prognosis were analyzed. Results: Of the 12 patients, 7 were male and 5 were female. The age of onset was (7.0±3.7)years (1.3 year to 13 years). The average hospitalization time (34-86 days, median 52 days). Twelve patients were healthy before the disease, and had fever before convulsion. The interval between fever and seizure was (3.5±1.7)days (1-7 days). The status epilepticus and consciousness deficit were the main clinical manifestations. The electrogram of 8 patients showed status epilepticus when admitted. 12 patients had disturbance of consciousness; the acute episodes were focal seizures (100%, 12/12) and generalized tonic-clonic seizures (41.7%, 5/12). All patients used 3-5 antiepileptic drugs (median 4), all treated with hormones and gamma globulin. 4 patients with ketogenic diet (KD) were treated within 2 weeks of onset, and the average duration from onset to electroencephalogram (EEG) improvement was (19.2±5.0)days. In 8 patients who did not use KD within 2 weeks of onset, the average duration from onset to EEG improvement was (29.9±9.6)days. Conclusions FIRES is more common in normal children with school age. The main manifestation is refractory status epilepticus in the days after acute fever, focal episodes of seizures, anti-epileptic drug resistance. Early initiation of KD produces a favorable prognosis.